title: Pediatric and adult lung transplantation for cystic fibrosis.
abstract: OBJECTIVE
  This paper was undertaken to review the experience at our institution with bilateral sequential lung transplantation for cystic fibrosis.
  
  
  METHODS
  Since 1989, 103 bilateral sequential lung transplants for cystic fibrosis have been performed (46 pediatric, 48 adult, 9 redo); the mean age was 21 +/- 10 years. Cardiopulmonary bypass was used in all but one pediatric (age <18) transplant, and in 15% of adults.
  
  
  RESULTS
  Hospital mortality was 4.9%, with 80% of early deaths related to infection. Bronchial anastomotic complications occurred with equal frequency in the pediatric and the adult populations (7.3%). One- and 3-year actuarial survival are 84% and 61%, respectively (no significant difference between pediatric and adult age groups; average follow-up 2.1 +/- 1.6 years). Mean forced expiratory volume in 1 second increased from 25% +/- 9% before transplantation to 79% +/- 35% 1 year after transplantation. Acute rejection occurred 1.7 times per patient-year, with most episodes taking place within the first 6 months after transplantation. The need for treatment of lower respiratory tract infections occurred 1.2 times per patient in the first year after transplantation. Actuarial freedom from bronchiolitis obliterans was 63% at 2 years and 43% at 3 years. Redo transplantation was performed only in the pediatric population and was associated with an early mortality of 33%. Eight living donor transplants (four primary transplants, four redo transplants) were performed with an early survival of 87.5%.
  
  
  CONCLUSIONS
  Patients with end-stage cystic fibrosis can undergo bilateral lung transplantation with morbidity and mortality comparable to that seen in pulmonary transplantation for other disease entities.
authors.affiliation: Department of Surgery, Washington University School of Medicine, St. Louis, Mo, USA.
authors.forename: E N
authors.initials: EN
authors.lastname: Mendeloff
authors.forename: C B
authors.initials: CB
authors.lastname: Huddleston
authors.forename: G B
authors.initials: GB
authors.lastname: Mallory
authors.forename: E P
authors.initials: EP
authors.lastname: Trulock
authors.forename: A H
authors.initials: AH
authors.lastname: Cohen
authors.forename: S C
authors.initials: SC
authors.lastname: Sweet
authors.forename: J
authors.initials: J
authors.lastname: Lynch
authors.forename: S
authors.initials: S
authors.lastname: Sundaresan
authors.forename: J D
authors.initials: JD
authors.lastname: Cooper
authors.forename: G A
authors.initials: GA
authors.lastname: Patterson
mesh_terms: D000206:Actuarial Analysis; D000293:Adolescent; D000328:Adult; D000890:Anti-Infective Agents / Q000627:therapeutic use; D002648:Child; D000075202:Contraindications; D003550:Cystic Fibrosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016040:Lung Transplantation / Q000379:methods / Q000401:mortality; D008297:Male; D008875:Middle Aged; D018579:Patient Selection; D012129:Respiratory Function Tests; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D000890:Anti-Infective Agents; D007166:Immunosuppressive Agents